• Proceedings of the KTCVS Conference
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• 1996.10a
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• pp.41-41
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• 1996

• Journal of Chest Surgery
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• v.31 no.9
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• pp.861-866
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• 1998

Background: Pulmonary atresia (PA) with ventricular septal defect has various morphology of pulmonary arteries and pulmonary blood flow sources, so pulmonary arterial hypoplasia and arborization abnormality make this anomaly difficult to manage surgically. In cases associated with juxtaductal stenosis, we evaluated the change of the pulmonary arterial and juxtaductal stenotic site after shunt operations, and would like to find useful information in surgical planning and methodology of these patients. Material and Method: Among 59 cases diagnosed as PA with ventricular septal defect associated with juxtaductal stenosis, 29 cases who had cardiac catheterization before and after shunt operation were selected from July, 1991 to July, 1996. In 10 cases of right shunt operation(Group I) and 19 cases of left shunt operation (Group II), the diameters of the descending aorta, both pulmonary arteries, and the juxtaductal stenosis site were measured before and after the shunt operation. Result: In both Group I and II, the pre- and postoperative ratio of diameters of the ipsilateral pulmonary artery to the descending aorta was from 0.78${\pm}$0.31 units to 1.01${\pm}$0.26 units and from 0.67${\pm}$0.18 units to 0.84${\pm}$0.27 units respectively, showing a signigicant increase. The contralateral pulmonary artery index was increased from 0.92${\pm}$0.28 units to 1.05${\pm}$0.15 units and from 0.94${\pm}$0.27 units to 1.08${\pm}$0.37 units respectively, but could not be confirmed statistically. In both groups, the change of juxtaductal stenosis showed an aggravating tendency but of no statistical significance from 0.43${\pm}$0.27 units to 0.39${\pm}$0.25 units and from 0.32${\pm}$0.10 units to 0.30${\pm}$0.16 units respectively, and we experienced 2 total obstruction in Group II. Because the increased pulmonary blood flow by shunt operation has a favorable effect to the pulmonary arterial growth, the shunt operation is a recommended treatment in patients with hypoplastic pulmonary arteries. But in PA with ventricular septal defects, the change of juxtaductal stenosis is very important. In conclusion, the growth of ipsilateral (shunt site) pulmonary artery was promoted by shunt operation, but there is a tendency for the juxtaductal stenosis to be aggravated. And we experienced 2 total obstruction in Group II. Conclusion: Thus, in cases operated with shunt method, much careful postoperative follow up study including angiographic evaluation is needed, and after the shunt operation on the side of pulmonary artery associated with juxtaductal stenosis, early precise planning for total correction is recommended.

• Journal of Chest Surgery
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• v.38 no.4 s.249
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• pp.284-290
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• 2005

We evaluated the early and mid-term results for repair of Ebstein's anomaly. Material and Method: Between January 1989 and June 2004, 29 patients underwent repair of Ebstein's anomaly. The median age was 11.4 years (4 days-50 years). Tricuspid insufficiency equal to or greater than grade 3 was present in 21 $(72.4\%)$ patients. Surgical techniques included tricuspid valve repair with vertical plication of the atrialized ventricle (n=14), Carpentier's technique (n=7), tricuspid valve replacement (n=4), systemic-to-pulmonary arterial shunt (n=2), tricuspid valve repair (n=1), and Fontan operation (n=1). Bi-directional cavopulmonary shunt (BCPS) was required in 5 patients. Among the 2 neonates, one patient underwent successful biventricular repair, and the other patient underwent systemic-to-pulmonary arterial shunt. Follow-up was possible in 21 patients $(75\%)$, and the average follow-up was 37.6 months (3 months-11.3 years). Result: There were $1(3.4\%)$ early and 1 late deaths. Reoperation was required in 4 patients. Two patients underwent tricuspid valve re-replacement, and the other 2 tricuspid valve repair. At recent follow-up, only 2 patients showed tricuspid insufficiency equal to or greater than grade 3, and most patients showed clinical improvement. Excluding the patients who underwent tricuspid valve replacement, the actuarial rate of freedom from reoperation at 1 and 5 years were $94.7\%\;and\;79.0\%$, respectively. Conclusion: Tricuspid valve repair was possible in most patients with good mid-term outcome. Most patients showed clinical and hemodynamic improvement. Indications for the BCPS should be clarified.

• Journal of Chest Surgery
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• v.30 no.11
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• pp.1121-1124
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• 1997

This is a report of a successful, rapid two-stage arterial switch operation in an infant with dextrotransposition of great arteries with an intact ventricular septum beyond the appropriate time for arterial switch operation.'A 4-month-old female infant was admitted due to severe cyanosis, respiratory insufficiency, and frequent dia rhea secondary to ischemic colitis. Echocardiographic examination presented marked leftward deviation of the interventricular septum, and left and right ventricular pressures as measured by cardiac catheterization were 40/4 mmHg and 85/2 mmHg, respectively. Fifteen days after a preparatory operation (pulmonary artery band with modified right Blalock-Taussig shunt), left ventricular-right ventricular systolic pressure ratio increased to 105/90. Arterial switch operation associated with the division of the right Blalock-Taussig shunt and the removal of pulmonary artery band was performed. A(leer the operation, the infant showed normal cardiac function with no postoperative complication.

• Journal of Chest Surgery
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• v.29 no.10
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• pp.1081-1089
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• 1996

Pulmonary atresia with intact ventricular septum has continued to have a high surgical mortality and morbidity. This mAy attribute to the non-uniformity of the anomaly. We reviewed a total of 34 infants with pulmonary atresla and intact ventricular septum managed in this hospital between 1987 and 1995. Mean age and body weight were 57.2 (range, 3-208) days and 4.1 (range, B.3∼6.8) kg. The preoperative Z-value of the diameter of the tricuspid valve was less than -2 in 85.2% of patients and less than -4 in 33. 3% . It is well correlated w th right ventricular cavity size (n=27. r10.68, p< 0.05). Coronary artery-right ventricular fistulas were identified in 3 patients, and right ventricular dependency was suspected in 1 Over All hospital mortality was 23.5%(8/34), although it decreased to 16.6%(4124) in 1990s. Subsequent procedures were performed in 6 patients between 3 days and 58 months after Initial palliation : one bidirectional cavopulmonary shunt and 1 Fontan operation after systemic-pulmonary shunt, 3 transannular patch + atrial septal defect closure and 1 additional systemic-pulmonary shunt after polmonary valvectomy or valvotomy. Changes of Z-values of the diameter of tricuspid valve have been followed up in 11 patients between 1 and 66 months postoperatively. Z-values were increased In 5 out of 8 transannular right ventriculAr outflow tract enlargement group and in 1 out of 3 pulmonary valvectomy or valvotomy group. Our data suggest that tailoring a treatment to right ventricular cavity size and coronary anom lies may improve the surgical outcome. A Z-value of the tricuspid valve diameter could be used.

• Journal of Chest Surgery
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• v.37 no.6
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• pp.539-542
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• 2004

Not only morphology of pulmonary artery or pulmonary artery resistance, but major aortopulmonary collateral arteries are risk factors of Fontan procedure. We report a successful Fontan procedure after rehabilitation of pulmonary arteries by unifocalization and systemic to pulmonary shunt in a high risk Fontan candidate with functional single ventricle combined with hypoplastic pulmonary arteries and major aortopulmonary collateral arteries supplying most of the bilateral lung field.

• Clinical and Experimental Pediatrics
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• v.52 no.4
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• pp.494-498
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• 2009

Purpose : Applicability of transcatheter closure of atrial septal defect (ASD) has been expanded by accumulation of clinical experiences and evolutions of the device. This study was performed to evaluate the safety and efficacy of transcatheter closure of ASD with Amplatzer septal occluder (ASO) in young children less than 3 years of age. Methods : From May 2003 to December 2005, 295 patients underwent transcatheter closure of ASD with ASO in the Severance Cardiovascular Hospital, Yonsei University Health System. Among them, 51 patients less than 3 years of age were enrolled in this study. We investigated procedural success rate, rate of residual shunt, frequency of complications, procedure/fluoroscopy time, and need of modified techniques for device implantation. Results : The median age was 2.1 years and median body weight was 12 kg. Implantation of device was successful in 50 patients (98%). Seven patients (15%) showed a small residual shunt 1 day after the procedure, but complete occlusion had been documented at 6 month follow-up in all patients (100%). The pulmonary to systemic flow ratio (Qp/Qs), peak systolic pulmonary artery pressure, and peak systolic right ventricular pressure had decreased significantly after closure of ASD. There were 2 complications including device embolization (1, 2%) and temporary groin hematoma (1, 2%). Conclusion : Transcatheter closure of ASD with ASO can be performed with satisfactory results and acceptable risk even in young children less than 3 years of age. We could suggest that even in very young children with ASD, there is no need to wait until they grow to a sufficient size for the transcatheter closure.

• Journal of Chest Surgery
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• v.38 no.6 s.251
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• pp.403-409
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• 2005

Pulmonary atresia with intact ventricular septum is morphologically heterogeneous, and the surgical outcome remains suboptimal compared to other complex congenital heart defects. We evaluated the long-term results for repair of pulmonary atresia with intact ventricular septum, Material and Method: Between January 1992 and June 2004, 38 patients underwent repair of pulmonary atresia with intact ventricular septum. The average age was 18 days $(2\~382\;days)$. The average Z-value of the tricuspid annulus diameter was -3.1$(-5.6\~0.8)$. Thirteen $(36\%)$ patients had right ventricle-to-coronary artery fistulas, and $4(11\%)$ patients had right ventricle-dependent coronary circulation. Average follow-up was 55 months $(3\;months\~2.2\;years)$, Result: Twenty-four patients under-went initial right ventricle (RV) decompression and 14 patients underwent systemic-to-pulmonary arterial shunt only. The average size of the tricuspid annulus of the patients who underwent RV decompression was significantly larger than that of the patients who underwent systemic-to-pulmonary arierial shunt only (Z-value -2.2 vs. -4.8, p=0.000). There were $5(13\%)$ early and 1 late deaths. Early deaths occurred in 3 patients who had undergone RV decompression, and in 2 patients who had undergone systemic-to-pulmonary arterial shunt only (p=1.0). Biventricular repair was achieved in $12(32\%)$ patients, single ventricular repair in $8(21\%)$, and one and a half ventricular repair in $4(l1\%)$ patients. Nine $(24\%)$ patients are waiting for the definitive repair. Kaplan-Meier survival at 5 and 8 years was $83.2\%$, respectively. Conclusion: Most of the deaths occurred after the initial palliation. Overall long-term survival was satisfactory. Early mortality should be reduced with careful preoperative evaluation and proper surgical strategy.

• Journal of Chest Surgery
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• v.40 no.10
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• pp.696-700
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• 2007

We report here on the midterm results after a Starnes operation for a severely symptomatic neonate with Ebstein's anomaly. A one-day-old baby presented with cyanosis and severe cardiomegaly. We peformed patch closure of the tricuspid valve with a central shunt after failure of tricuspid valve repair with vertical plication of the atrialized ventricle at her age of 19 days. The coronary sinus was drained into the right ventricle. She underwent bidirectional cavopulmonary shunt and extracardiac conduit Fontan operation at her age of 16 and 30 months, respectively. She is now 56 months old and is doing very well. The recent follow-up study revealed that she was in normal sinus rhythm and had a normal sized left ventricle with good function and the small right ventricle without thrombus formation.

• Journal of Chest Surgery
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• v.29 no.8
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• pp.836-843
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• 1996

Histologic, and scanning electron microscopic observa ions were made of 12 biopsy specimens from polytetrdiluoroethylene (PTFE) grafts that had been Implanted as systemic-pulmonAry shunt for improving oxygenation of cyanotic congenital heart disease and harvested near the end of pulmonary artery side at the time of redo shunt or tonal correction between 1985 and 1992. The types of shunt operation are modified Blalock-Taussig shunt in 10 cases, Waterston-Cooley shunt in 1 And Potts shunt in 1 case. The 5 mm PTFE graft was used In 10, 4mm in 1 and 6mm in 1 case. The mean duration of implantalion was 0 $\pm$ 14.1 months(rl.on 12 months to 55 months). The plAtelets were aggregated between gaps'of Coretex surface and intimal thickening was noted about 10 to 20 months after implantation. Endothelial cells were found in the 40-months patent PTFE graft by light and scanning electron microscopy. In the specimen of poor flow or zero flow graft, severe intimal hyperplasia and thrombi which was made of platelets were noted . Based on this experience, we think that the patency will be maintained well if the connective tissue could be Hxed firmly over the Inner layer of the Goretex and the endothelial cell layer sllould form over the con- nective tissue and platelet aggregation should be prevented.